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Flashcards in CF Deck (7):
1

What is the mutation that causes of CF?
What is the cause of this mutation?

deletion of delta508 in CFTR gene on chrom 7q

defective CFTR Cl- channel

2

How does a baby with CF present?

-salty skin
-meconium illius (no abnormalities with GI tract, poop just gets stuck due to super viscous pancreatic secretions)
-failure to thrive

3

What causes all the problems in CF?

thick mucous secretions createes plugs in lungs, pancreas, and liver

4

What problems do CF pts have?

chronic bronhcitis --> bronchiectasis
pancreatic insufficiency --> FATTY REPLACEMENT
billiary cirrhosis
fat soluble vitamin def
infertility in men

5

How is CF diagnosed?

high Cl- conc in sweat test

6

What pneumonia is assc with CF?

pneudomonas and s.aureus

7

What is the treatment for CF?

N-Acetylcysteine loosens plugs by cleaving disulfide bonds in mucous glycoproteins